Entry
Name
Complement and coagulation cascades - Homo sapiens (human)
Description
The complement system is a proteolytic cascade in blood plasma and a mediator of innate immunity, a nonspecific defense mechanism against pathogens. There are three pathways of complement activation: the classical pathway, the lectin pathway, and the alternative pathway. All of these pathways generate a crucial enzymatic activity that, in turn, generates the effector molecules of complement. The main consequences of complement activation are the opsonization of pathogens, the recruitment of inflammatory and immunocompetent cells, and the direct killing of pathogens. Blood coagulation is another series of proenzyme-to-serine protease conversions, culminating the formation of thrombin, the enzyme responsible for the conversion of soluble fibrinogen to the insoluble fibrin clot. Protease-activated receptors, such as those activated by thrombin, are members of G protein-coupled receptors and function as a mediator of innate immunity. The kallikrein-kinin system is an endogenous metabolic cascade, triggering of which results in the release of vasoactive kinins (bradykinin-related peptides). Kinin peptides are implicated in many physiological and pathological processes including the regulation of blood pressure and sodium homeostasis, inflammatory processes, and the cardioprotective effects of preconditioning.
Class
Organismal Systems; Immune system
BRITE hierarchy
Pathway map
hsa04610 Complement and coagulation cascades
Ortholog table
Network
nt06136 Complement activation (viruses)
nt06164 Kaposi sarcoma-associated herpesvirus (KSHV)
nt06168 Herpes simplex virus 1 (HSV-1)
nt06171 Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2)
Element
N00463 Alternative pathway of complement activation
N01311 Lectin pathway of complement activation
N01313 Classical pathway of complement activation
N01315 Lectin pathway of coagulation activation
Drug
D00160 Aminocaproic acid (USP/INN)
D01136 Tranexamic acid (JP18/USP/INN)
D01672 Gabexate mesilate (JP18)
D01844 Fondaparinux sodium (JAN/USP/INN)
D01981 Ximelagatran (JAN/USAN/INN)
D02112 Heparin sodium (JP18/USP/INN)
D02235 Desmopressin acetate (USP)
D02956 Anticoagulant heparin (USP)
D02980 Adomiparin sodium (USAN)
D03213 Apixaban (JAN/USAN/INN)
D03330 Kallidinogenase (JP18/INN)
D03353 Dalteparin sodium (JAN/USP/INN)
D03644 Danaparoid sodium (JAN/USAN)
D03673 Deligoparin sodium (USAN/INN)
D03674 Enoxaparin sodium (JAN/USP/INN)
D03722 Efegatran sulfate (USAN)
D04029 Razaxaban hydrochloride (USAN)
D04492 Icatibant acetate (JAN/USAN)
D04977 Parnaparin sodium (JP18)
D06398 Tinzaparin sodium (USAN)
D06410 Thrombomodulin alfa (USAN/INN)
D07082 Dabigatran etexilate mesylate (USAN)
D07086 Rivaroxaban (JAN/USAN/INN)
D07144 Dabigatran etexilate (USAN/INN)
D07568 Aminomethylbenzoic acid
D09546 Edoxaban tosilate hydrate (JAN)
D09766 Vorapaxar sulfate (JAN/USAN)
D09817 Darexaban maleate (JAN)
D09867 Atopaxar hydrobromide (USAN)
D10150 Pegnivacogin sodium (USAN)
D10418 Delparantag pentahydrochloride (USAN)
D10471 Idraparinux sodium (USAN)
D10758 Turoctocog alfa pegol (genetical recombination) (JAN)
D11029 Andexanet alfa (USAN/INN)
D11093 Avacopan (JAN/USAN/INN)
D11160 Caplacizumab (USAN/INN)
D11280 Bivalirudin trifluoroacetate (USAN)
D11568 Edoxaban tosylate (USAN)
D11613 Pegcetacoplan (JAN/USAN/INN)
D11641 Danicopan (JAN/USAN/INN)
D11674 Berotralstat hydrochloride (JAN/USAN)
D11748 Avacincaptad pegol sodium (USAN)
D11814 Tisotumab vedotin (USAN/INN)
D12252 Iptacopan hydrochloride (USAN)
D12356 Zilucoplan sodium (JAN)
Organism
Homo sapiens (human) [GN:
hsa ]
Gene
2152 F3; coagulation factor III, tissue factor [KO:K03901 ]
5624 PROC; protein C, inactivator of coagulation factors Va and VIIIa [KO:K01344 ] [EC:3.4.21.69 ]
2149 F2R; coagulation factor II thrombin receptor [KO:K03914 ]
2151 F2RL2; coagulation factor II thrombin receptor like 2 [KO:K04235 ]
9002 F2RL3; F2R like thrombin or trypsin receptor 3 [KO:K04236 ]
2165 F13B; coagulation factor XIII B chain [KO:K03906 ]
623 BDKRB1; bradykinin receptor B1 [KO:K03915 ]
624 BDKRB2; bradykinin receptor B2 [KO:K03916 ]
7035 TFPI; tissue factor pathway inhibitor [KO:K03909 ]
462 SERPINC1; serpin family C member 1 [KO:K03911 ]
3053 SERPIND1; serpin family D member 1 [KO:K03912 ]
5104 SERPINA5; serpin family A member 5 [KO:K03913 ]
5054 SERPINE1; serpin family E member 1 [KO:K03982 ]
5055 SERPINB2; serpin family B member 2 [KO:K19821 ]
5329 PLAUR; plasminogen activator, urokinase receptor [KO:K03985 ]
5265 SERPINA1; serpin family A member 1 [KO:K03984 ]
5345 SERPINF2; serpin family F member 2 [KO:K03983 ]
2 A2M; alpha-2-macroglobulin [KO:K03910 ]
731 C8A; complement C8 alpha chain [KO:K03997 ]
732 C8B; complement C8 beta chain [KO:K03998 ]
733 C8G; complement C8 gamma chain [KO:K03999 ]
5648 MASP1; MBL associated serine protease 1 [KO:K03992 ] [EC:3.4.21.-]
720 C4A; complement C4A (Rodgers blood group) [KO:K03989 ]
721 C4B; complement C4B (Chido blood group) [KO:K03989 ]
719 C3AR1; complement C3a receptor 1 [KO:K04009 ]
11326 VSIG4; V-set and immunoglobulin domain containing 4 [KO:K19822 ]
1378 CR1; complement C3b/C4b receptor 1 (Knops blood group) [KO:K04011 ]
1379 CR1L; complement C3b/C4b receptor 1 like [KO:K04011 ]
728 C5AR1; complement C5a receptor 1 [KO:K04010 ]
710 SERPING1; serpin family G member 1 [KO:K04001 ]
1604 CD55; CD55 molecule (Cromer blood group) [KO:K04006 ]
722 C4BPA; complement component 4 binding protein alpha [KO:K04002 ]
725 C4BPB; complement component 4 binding protein beta [KO:K04003 ]
3078 CFHR1; complement factor H related 1 [KO:K23815 ]
3080 CFHR2; complement factor H related 2 [KO:K23815 ]
966 CD59; CD59 molecule (CD59 blood group) [KO:K04008 ]
Compound
Reference
Authors
Bajic G, Degn SE, Thiel S, Andersen GR
Title
Complement activation, regulation, and molecular basis for complement-related diseases.
Journal
Reference
Authors
Mathern DR, Heeger PS
Title
Molecules Great and Small: The Complement System.
Journal
Reference
Authors
Merle NS, Church SE, Fremeaux-Bacchi V, Roumenina LT
Title
Complement System Part I - Molecular Mechanisms of Activation and Regulation.
Journal
Reference
Authors
Oikonomopoulou K, Ricklin D, Ward PA, Lambris JD
Title
Interactions between coagulation and complement--their role in inflammation.
Journal
Reference
Authors
Kurosawa S, Stearns-Kurosawa DJ
Title
Complement, thrombotic microangiopathy and disseminated intravascular coagulation.
Journal
Reference
Authors
Hillmeister P, Persson PB
Title
The Kallikrein-Kinin system.
Journal
Reference
Authors
Rau JC, Beaulieu LM, Huntington JA, Church FC
Title
Serpins in thrombosis, hemostasis and fibrinolysis.
Journal
Reference
Authors
Crooks MG, Hart SP
Title
Coagulation and anticoagulation in idiopathic pulmonary fibrosis.
Journal
Reference
Authors
Christiaans SC, Wagener BM, Esmon CT, Pittet JF
Title
Protein C and acute inflammation: a clinical and biological perspective.
Journal
Reference
Authors
Alberelli MA, De Candia E
Title
Functional role of protease activated receptors in vascular biology.
Journal
Reference
Authors
Smith HW, Marshall CJ
Title
Regulation of cell signalling by uPAR.
Journal
Reference
Authors
Ricklin D, Lambris JD
Title
Complement in immune and inflammatory disorders: pathophysiological mechanisms.
Journal
Reference
Authors
Ricklin D, Lambris JD
Title
Complement in immune and inflammatory disorders: therapeutic interventions.
Journal
Reference
Authors
Loof TG, Deicke C, Medina E
Title
The role of coagulation/fibrinolysis during Streptococcus pyogenes infection.
Journal
Reference
Authors
Jozsi M, Tortajada A, Uzonyi B, Goicoechea de Jorge E, Rodriguez de Cordoba S
Title
Factor H-related proteins determine complement-activating surfaces.
Journal
Related pathway
hsa04662 B cell receptor signaling pathway
hsa04750 Inflammatory mediator regulation of TRP channels
KO pathway
LinkDB
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